Sco1 Gene Summary [Mouse]
Predicted to enable metal ion binding activity. Acts upstream of or within several processes, including hematopoietic or lymphoid organ development; intracellular monoatomic cation homeostasis; and regulation of intracellular signal transduction. Located in mitochondrion. Is expressed in several structures, including cardiovascular system; genitourinary system; hemolymphoid system gland; liver; and submandibular gland primordium. Used to study cytochrome-c oxidase deficiency disease. Human ortholog(s) of this gene implicated in cytochrome-c oxidase deficiency disease and mitochondrial complex IV deficiency nuclear type 4. Orthologous to human SCO1 (synthesis of cytochrome C oxidase 1). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Sco1 |
| Official Name | SCO1 cytochrome c oxidase assembly protein [Source:MGI Symbol;Acc:MGI:106362] |
| Ensembl ID | ENSMUSG00000069844 |
| Bio databases IDs | |
| Aliases | SCO1 cytochrome c oxidase assembly protein |
| Synonyms | 2610001C07Rik, D11Bwg1310e, MC4DN4, RGD1559538, SCO1 cytochrome c oxidase assembly protein, SCOD1, synthesis of cytochrome C oxidase 1 |
| Species | Mouse, Mus musculus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Sco1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- Thioredoxin-like
- Protein Disulfide Oxidoreductases and Other Proteins with a Thioredoxin fold
- SCO1/SenC
- AhpC/TSA family
- protein binding
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- nuclear type 4 mitochondrial complex IV deficiency
- mitochondrial disorder
- nuclear type 1 mitochondrial complex iv deficiency
regulated by
- TP53
- beta-estradiol
- OGT
- myoblasts
- NEF
- CD3 (complex)
regulates
- lipid
- iron ion
- Cu2+
- PGAM5
- Zn2+
role in cell
- organization
- differentiation
- homeostasis in
- molecular cleavage in
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- Mitochondria
- mitochondrial inner membrane
- myofibrils
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Sco1 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- mitochondrial respiratory chain complex IV assembly
- cellular copper ion homeostasis
Cellular Component
Where in the cell the gene product is active
- mitochondrion
- mitochondrial inner membrane
- myofibril
Molecular Function
What the gene product does at the molecular level
- protein binding
- copper ion binding
- copper chaperone activity